Alshammari et al.

Journal of Medical Case Reports 2012, 6:410

http://www.jmedicalcasereports.com/content/6/1/410 JOURNAL OF MEDICAL
CASE REPORTS

CASE REPORT Open Access

Clival chordoma with an atypical presentation: a

case report
Jaber Alshammari1, Philippe Monnier1, Roy T Daniel2 and Kishore Sandu1,3*

Abstract
Introduction: Clival chordomas present with headache, commonly VI cranial nerve palsy or sometimes with lower
cranial nerve involvement. Very rarely, they present with cerebrospinal fluid rhinorrhoea due to an underlying
chordoma-induced skull base erosion.
Case presentation: A 60-year old Caucasian woman presented with meningitis secondary to cerebrospinal fluid
rhinorrhoea. At first, radiological imaging did not reveal a tumoral condition, though intraoperative exploration and
tissue histology revealed a chordoma which eroded her clivus and had a transdural extension.
Conclusion: Patients who present with meningitis and cerebrospinal fluid rhinorrhoea could have an underlying
erosive lesion which can sometimes be missed on initial radiological examination. Surgical exploration allows
collecting suspicious tissue for histological diagnosis which is important for the actual treatment. A revision
endoscopic excision of a clival chordoma is challenging and has been highlighted in this report.

Introduction case report of a patient who presented initially with menin-

Chordomas are rare, slow-growing malignant bony tu-
mors that arise from remnants of the notocord and ac-
count for approximately 1% of intracranial tumors [1].
Approximately 35% to 40% of these tumors occur in the
skull base, where they typically involve the clivus [1-3].
While these tumors rarely metastasize to distant sites,
they are locally aggressive and tend to recur after surgi-
cal resection, with a life expectancy of less than 10 years
after diagnosis [4]. Most commonly, the patient presents
with headache, diplopia secondary to VI cranial nerve
paresis and visual changes including blurring or some-
times loss of vision [5]. The patient may present with
multiple lower cranial nerve palsy symptoms such as
facial numbness and asymmetry, dysphagia, hoarseness
and speech problems. Finally, large tumors may cause
brainstem compression and patients may present with
long tract signs and ataxia [6,7]. Epistaxis as a rare pre-
sentation has also been reported [8].
Cerebrospinal fluid (CSF) rhinorrhea has been very rarely
described as a presenting symptom [9,10]. We present a
* Correspondence: kishore.sandu@hopitalvs.ch
1
Department of Otorhinolaryngology, University Hospital - CHUV,
Lausanne, Switzerland
3
Department of Otorhinolaryngology, Valais State Hospital - CHCV,
Sion, Switzerland
Full list of author information is available at the end of the article
© 2012 Alshammari et al.; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the
Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use,
distribution, and reproduction in any medium, provided the original work is properly cited.
gitis and later was diagnosed to have a clival chordoma.
We discuss the treatment modalities and present a syste-
matic review of the literature.
Case presentation
A 60-year-old Caucasian woman, living alone and pre-
viously in good health, was found unconscious at home
and fortunately helped by her friends and the emergency
medical team. One week before this incident, she had
infrequent episodes of watery nasal discharge that in-
creased with forced sneezing and cough. On her arri-
val to our clinic, she was confused and febrile. A cranial
nerve examination was normal. A computed tomography
(CT) scan (Figure 1) of her brain and paranasal sinuses
showed pneumocephalus in the ventricles, hydrocephalus
and an air-fluid level in the right sphenoid sinus indicating
a CSF leak. There was a bony defect of the clivus with
sclerotic edges. A lumbar puncture and analysis of her
CSF confirmed bacterial meningitis and our patient was
immediately started on ceftazidime 2g three times a day.
Magnetic resonance imaging (MRI) (Figures 2 and 3)
showed an osteolytic, irregular lesion occupying the
upper clivus and measuring 13×12×16mm. It was hype-
rintense on T2-weighted images and hypointense on
T1-weighted images. The lesion showed no enhancement
Alshammari et al. Journal of Medical Case Reports 2012, 6:410
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Figure 1 Computed tomography scan showing presence of air
in the subarachnoid spaces and ventricles.
with administration of gadolinium. An endonasal trans-
sphenoidal endoscopic exploration of the skull base defect
and closure of the CSF fistula was planned. A bony defect
and CSF leak was visualized on the postero-inferior wall
of her right sphenoid sinus at the posterior cortical margin
of her clivus. Further exploration revealed suspicious
Figure 2 T2-weighted sagittal images showing erosion of the
posterior wall of the clivus and hyperintensity suggestive of
cerebral spinal fluid, with a fluid level in the sphenoid sinus.
Note also, the presence of pneumocephalus with air in the
subarachnoid spaces and the ventricle.
Page 2 of 5
Figure 3 T1-weighted sagittal images showing an osteolytic
lesion of the clivus with no contrast enhancement.
tissue adjacent to the dural defect that was just anterior to
her basilar artery. This tissue was carefully dissected and
sent for histopathological examination. The skull base de-
fect was repaired using fascia lata and the sphenoid sinus
was obliterated with fat and fibrin glue TISSEELW. Histo-
pathologic analysis of the biopsied tissue showed dark
giant oval nuclei, with vacuolated or granular eosinophilic
cytoplasm. The cells were arranged in epithelial cords,
and on immunohistochemistry were strongly positive for
S-100 protein and epithelial membrane antigen, thereby
confirming diagnosis of a chordoma in the clival region.
Three months later, our patient underwent an MRI
(Figures 4 and 5) to re-define the presence and extent of
the clival chordoma. In the intervening period she was
asymptomatic and did not have any CSF leak. A transnasal
endoscopic resection of the chordoma aided by neuro-
navigation was planned. Surgery began with a complete
right-side ethmoidectomy. Posterior septectomy allowed a
'two nostrils - four hands’ technique for tumor removal. A
radical vomerectomy was performed. The entire anterior
wall of her sphenoid sinus and her sphenoidal septum
were excised. During a revision endoscopic exploration it
is extremely important to identify the bony edges circum-
ferentially around the earlier defect and gradually define
the bone and soft tissue junction, which necessitated re-
moval of the upper two-thirds of her clivus. The next
step was to identify the sella, optic nerve and the caro-
tid impressions bilaterally within her sphenoid sinus. Sub-
sequently, the normal dura and fibrocicatricial tissue
surrounding the chordoma were identified. An en bloc
excision of the chordoma was done, anticipating her
basilar artery to be just posterior to the tumor. Recons-
truction of the defect was performed using fascia lata, fat
Alshammari et al. Journal of Medical Case Reports 2012, 6:410
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Figure 4 T2-weighted sagittal images showing a predominantly
hyperintense lesion involving the posterior wall of the clivus just
anterior to the basilar artery. The sphenoid sinus shows evidence
of packing done during the first surgery with inflammatory changes.
and fibrin glue. A lumbar drain was inserted preopera-
tively and used to drain CSF in the postoperative period.
It was removed on the fifth postoperative day. Broad spec-
trum antibiotics (ceftazidime 2g three times a day) were
Figure 5 T1-weighted gadolinium enhanced images show a
hypointense lesion of the clivus more towards the right side
with destruction of the bony margins of the clivus. The lesion is
found just anterior to the basilar artery. There was no contrast
enhancement of the lesion.
Page 3 of 5
Figure 6 T2-weighted sagittal magnetic resonance scan shows
total excision of the lesion with the packing material seen in
the posterior part of the clivus in the extradural plane.
given for 10 days. Our patient had an excellent recovery
with no complications. The follow-up scans showed com-
plete excision of the tumor and no other adjuvant therapy
was instituted. Our patient was discharged on the 12th
postoperative day and a systematic clinico-radiological fol-
low-up is planned. She was totally asymptomatic at the
latest follow-up (three months after surgery) with an MRI
(Figures 6 and 7) showing complete excision of the tumor.
Figure 7 T1-weighted fat suppressed images show no evidence
of residual or recurrent tumor.
Alshammari et al. Journal of Medical Case Reports 2012, 6:410
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Discussion
Chordomas were first described by Virchow in 1857
as tumors made up of vacuolated or ‘physaliferous’ cells
derived from rests of embryonic notocord along the
midline central nervous system axis [11]. Chordomas can
invade and metastasize to bony structures like the sacro-
coccyx, skull base and vertebrae [11]. The histological
appearance of a chordoma includes pleomorphic cells
with dark nuclei and vacuolated or granular cytoplasm set
within an abundant myxoid matrix. Neoplastic cells are
arranged in epithelial cords separated by mucinous mate-
rial, which is a classic feature of chordomas. On immuno-
histochemistry, the cells are positive for S-100 protein and
epithelial membrane antigen [8]. The symptoms of a clival
chordoma depend mainly on the site of the tumor and the
adjacent structures. Headache, visual changes and cranial
nerve palsies are the most frequent symptoms [4], though
rare presentations like CSF rhinorrhea and epistaxis have
also been reported [8,10].
For diagnosis, it is mandatory to do a CT scan and MRI
for all skull base tumors, although there are no reliable
diagnostic features that allow differentiation between these
tumors [11]. Generally, MRI is better for defining the
exact position of the brainstem and the optic chiasma
relative to the tumor with added information about tumor
extension into the nasopharynx and cavernous sinus. It
also demonstrates the position of the cavernous internal
carotid, vertebral and basilar arteries in relation to the
tumor [12]. CT is better than MRI in demonstrating tu-
moral calcification and associated bone destruction.
Clival chordomas can be managed by a variety of conven-
tional surgical approaches: transcranial, transsphenoidal,
transoropharyngeal and maxillary osteotomy approaches
[13]. Transcranial approaches involve brain retraction and
have increased risks of cerebral edema and hematoma,
apart from carotid, basilar artery and optic nerve trauma.
These complications can be greatly reduced with anterior
(transnasal, transoral and transfacial) approaches [13]. Cur-
rently, endoscopic surgery has opened a new avenue in the
management of clival chordomas, not only as a direct sur-
gical access but also by providing an excellent visualization
of the clivus and surrounding structures, especially the an-
terior dura and the basilar artery. The surgical procedures,
techniques and selection of the endoscopic surgical stra-
tegies for preservation of the vital anatomic structures are
described in detail in the literature [13].
This case report is interesting on several counts. In ge-
neral, patients with clival chordomas present with head-
ache or cranial nerve palsies. Our patient was hospitalized
as an emergency case with meningitis, which in turn was
secondary to a CSF leak caused by skull base erosion.
MRI showed an osteolytic, irregular lesion occupying
her upper clivus that was hyperintense on T2-weighted
images and hypointense on T1-weighted images, showing
Page 4 of 5
no enhancement with administration of gadolinium. Nei-
ther CT nor MRI images revealed any obvious tumor
mass. Transnasal endoscopic exploration allowed us to
close the skull base defect and collect local soft tissue for
histological analysis, which showed evidence of a chor-
doma. A second surgery for an en bloc resection of the
chordoma was performed three months after the initial
intervention. It is quite possible that, right at the first pres-
entation, our patient had a clival chordoma with skull base
erosion, but it did not enhance on contrast administration
and hence could not be diagnosed in the earlier CT and
MRI scans. An intradural extension of the tumor led to a
CSF leak and subsequent meningitis. A systematic explo-
ration of her skull base allowed us to send local soft tissue
for histological analysis, which confirmed the diagnosis of
a chordoma and allowed us to plan the subsequent radical
endoscopic excision.
A revision endoscopic skull base surgery can be diffi-
cult due to severe postoperative fibrocicatricial tissue as
a result of the packing material (fat, fascia lata) used for
the CSF leak closure and skull base reconstruction du-
ring the prior surgery. During such revision explorations,
it is of paramount importance to define the tumoral soft
tissue and bone erosion limits, followed by identification
of the important landmarks within the sphenoid sinus.
Neuronavigation helps in identifying vital anatomical
structures at the anterior skull base and avoids intraopera-
tive complications, thereby facilitating a complete tumor
removal. The two nostrils - four hands technique facili-
tates better transnasal instrumentation allowing a com-
plete tumor excision and efficient reconstruction of the
skull base defect.
The role of radiation therapy has been extremely con-
troversial in the treatment of clival chordomas. Con-
ventional radiation does not appear to have an effect on
survival in the study published by Colli and Al-Mefty
[14]. Results of this study indicated that the patient over-
all survival significantly improved with a radical resec-
tion of the tumor combined with postoperative proton
beam radiotherapy. There is no study in the literature
comparing the results of surgery alone and surgery with
postoperative radiotherapy. Finally, with a limited number
of cases in the literature, we cannot conclude on the most
effective and ideal management of these cases, though
most authors agree that complete surgical excision of the
tumor mass with an adequate margin provides the best
chance for a recurrence-free survival [14,15].
Conclusion
Patients with a clival chordoma commonly present with
headaches, visual changes, cranial nerve palsies and, rare-
ly, with CSF rhinorrhea and epistaxis. Patients who pre-
sent with meningitis secondary to a CSF leak may have
an underlying erosive lesion that can be missed on initial
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radiological examination. A revision transnasal endoscopic
excision of a clival chordoma is challenging and warrants
collaborative efforts between the ear, nose and throat sur-
geons and neurosurgeons.
Consent
Written informed consent was obtained from the patient
for publication of this manuscript and accompanying
images. A copy of the written consent is available for re-
view by the Editor-in-Chief of this journal.
13. Hong Jiang W, Ping Zhao S, Hai Xie Z, Zhang H, Zhang J, Yun Xiao J:
Endoscopic resection of chordomas in different clival regions.
Acta Otolaryngol 2009, 129:71–83.
14. Colli B, Al-Mefty O: Chordomas of the craniocervical junction: follow-up
review and prognostic factors. J Neurosurg 2001, 95:933–943.
15. Raffel C, Wright DC, Gutin PH, Wilson CB: Cranial chordomas: clinical
presentation and results of operative and radiation therapy in twenty-six
patients. Neurosurgery 1985, 17:703–710.
doi:10.1186/1752-1947-6-410
Cite this article as: Alshammari et al.: Clival chordoma with an atypical
presentation: a case report. Journal of Medical Case Reports 2012 6:410.

Competing interests
The authors declare that they have no competing interests.

Authors’ contributions
JA - collection of data, manuscript writing. PM - analysis and editing.
RTD - analysis and editing the manuscript. KS - collection of data, manuscript
writing, analysis and editing the manuscript. KS was the major contributor in
writing the manuscript. All authors read and approved the final manuscript.

Author details
1
Department of Otorhinolaryngology, University Hospital - CHUV,
Lausanne, Switzerland. 2Department of Neurosurgery, University Hospital
- CHUV, Lausanne, Switzerland. 3Department of Otorhinolaryngology,
Valais State Hospital - CHCV,
Sion, Switzerland.

Received: 12 July 2012 Accepted: 16 October 2012

Published: 29 November 2012

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